系统性红斑狼疮合并血栓性血小板减少性紫癜的临床特点
2013-05-10 晓静 整理 医学论坛网
中国医学科学院北京协和医学院崔静、朱铁楠和张奉春等探讨了系统性红斑狼疮(SLE)合并血栓性血小板减少性紫癜 (TTP) 的临床特点、诊断及治疗。结果发现,SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要。SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后。相关论文在
中国医学科学院北京协和医学院崔静、朱铁楠和张奉春等探讨了系统性红斑狼疮(SLE)合并血栓性血小板减少性紫癜 (TTP) 的临床特点、诊断及治疗。结果发现,SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要。SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后。相关论文在线发表于2013年第5期《中华内科杂志》上。
研究者们回顾性分析了14例SLE合并TTP者的临床表现、实验室检查结果、诊治和转归。结果显示,14例SLE合并TTP者中,男4例,女10例,中位年龄23(17~69)岁。5例以SLE首发,9例两者同时发病。所有患者均有血小板减少、微血管病性溶血性贫血,发热、神经系统异常、肾功能损害的发生比例分别为12/14、11/14、11/14,其中8例患者具有上述五联征。糖皮质激素(单用或联合静脉注射人免疫球蛋白和环磷酰胺)治疗6例,糖皮质激素联合血浆置换8例,有效率分别为2/6、6/8。8例患者存活,6例死亡。对6例存活者进行了随访,均无复发。
与红斑狼疮相关的拓展阅读:
- AJHG:全基因组关联Meta分析搜寻系统性红斑狼疮易感基因
- Arthr Res & Ther:来那度胺或成为治疗皮肤红斑狼疮的有效疗法
- Lupus:血清肿瘤坏死因子-α水平与系统性红斑狼疮患者的生活质量和抑郁症状相关
- Blood:红斑狼疮治疗药不增加淋巴瘤风险 更多信息请点击:有关红斑狼疮更多威廉亚洲官网
The clinical analysis of 14 cases of systemic lupus erythematosus complicated by thrombotic thrombocytopenic purpura
Abstract: Objective To analyze the clinical features, diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE).Methods Clinical manifestations, laboratory findings, diagnosis,treatment and prognosis of 14 SLE patients with TTP were retrospectively analyzed.Results Of the 14 patients diagnosed with SLE and TTP, 4 were men and 10 were women. The median age at diagnosis was 23(17-69) years old. In five patients, the onset of SLE preceded TTP, and in nine patients SLE and TTP occurred simultaneously. All the 14 patients had thrombocytopenia and hemolytic anemia,12 had fever, 11 had neurologic abnormalities, and 11 had renal dysfunction. Eight patients presented with the classic pentad of symptoms. Six patients were given steroids (alone or in combination with intravenous immunoglobulin and cyclophosphamide), and eight patients were treated with steroids in combination with plasmapheresis, with response rates of 2/6 and 6/8, respectively. Six patients died, with overall mortality rate of 6/14. No patients relapsed during the follow-up period.ConclusionsSLE and TTP share some similar clinical symptoms. As a result, repeated examinations of peripheral blood smears are very important for early diagnosis. The renal damage in patients of co-existing diseases is more serious than those with TTP alone or SLE alone. Early diagnosis and prompt treatment with plasma exchange and steroids may improve the prognosis in SLE patients with TTP.
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在此留言
#临床特点#
58
#血小板减少性紫癜#
60
#血栓性#
50
Great article, totally what I wanted to find.
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#系统性#
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#血小板减少#
48
#狼疮#
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#红斑#
53
#紫癜#
57
#血栓性血小板减少性紫癜#
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